Severe Ocular Cicatricial Pemphigoid – A Sad Surface Plus Surgery Equals Scarring

Ocular cicatricial pemphigoid (OCP) can be notoriously aggressive and is an important and common cause of cicatrising conjunctivitis in the UK, around 60% of cases can be attributed. Even untreated disease progresses in up to 75% of patients despite the eyes appearing quiet and white.

It is characterised by bilateral chronic autoimmune relapsing-remitting conjunctivitis which may lead to visual loss secondary to cicatricial changes.

The following case is an illustration of the difficulties in managment of severe ocular surface disease secondary to OCP and how surgical manipulation and augmentation using amniotic membrane graft can lead to a vicious cycle, ending in blinding disease.

Ophthalmology Unit, University Hosptials Coventry and Warwickshire, Coventry, UK

An 83-year-old female presented to eye casualty with a 2-week history of foreign body sensation, watering, redness and soreness in the right eye. On examination symblephara and forniceal shortening were seen. Visual acuity was 6/7.5 RE and 6/5 LE. A suspicion of OCP led to rheumatological and dermatological review and conjunctival biopsy, which was positive. 

Treatment was initiated with topical and oral steroid, topical levofloxacin, topical tacrolimus 0.1% and lubricants At follow up, vision in the right eye had reduced to hand movements. 10 cyclophosphamide infusions were performed over 5 months.

5 months post biopsy, the RE had stage 4 OCP, with complete obliteration of the inferior fornix and inferior corneal keratinisation. The LE had remained stable at stage 2.

Total forniceal reconstruction using buccal mucus membrane graft and amniotic membrane transplantation (AMT) was performed and allogenic serum eye drops prescribed.

Postoperatively, symblephara persisted. Mycophenolate mofetil (MMF) was added and two further reconstructions with AMT were done over the next 6 months.

End stage OCP with cicatricial entropion, trichiasis, persistent epithelial defect and later, limbal stem cell deficiency developed. Visual acuity remained hand movements in the RE. The LE had VA 6/24 with cataract.

A final fornix reconstruction with AMT in right eye and simultaneous left eye cataract surgery augmented with AMT was done resulting in 6/6 vision in a quiet left eye.

OCP can be an aggressive disease. Conservative measures may sometimes be the best course of action due to the vicious cyclical nature of the condition leading only to scarring. Systemic immunosuppression remains the mainstay of treatment. Asymmetric disease is not uncommon. Severe dry ocular surface can indeed compromise surgery and such patients should avoid having any graft procedure to help in fornix reconstruction. Cataract surgery has favourable outcomes in patients who are stable on systemic therapy